Molybdopterin (BioDeep_00001869546)
Main id: BioDeep_00000006000
代谢物信息卡片
Molybdopterin
化学式: C10H14N5O6PS2 (395.01231140000004)
中文名称:
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: C(C1C(=C(C2C(O1)NC3=C(N2)C(=O)NC(=N3)N)S)S)OP(=O)(O)O
InChI: InChI=1S/C10H14N5O6PS2/c11-10-14-7-4(8(16)15-10)12-3-6(24)5(23)2(21-9(3)13-7)1-20-22(17,18)19/h2-3,9,12,23-24H,1H2,(H2,17,18,19)(H4,11,13,14,15,16)/t2-,3+,9-/m1/s1
数据库引用编号
9 个数据库交叉引用编号
- ChEBI: CHEBI:44074
- KEGG: C05924
- PubChem: 135398581
- CAS: 220399-50-8
- PubChem: 8212
- KNApSAcK: C00000774
- PDB-CCD: MTE
- 3DMET: B05139
- NIKKAJI: J1.100.870D
分类词条
相关代谢途径
Reactome(9)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
0 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Simon Julius Mayr, Jörn Oliver Sass, Julia Vry, Janbernd Kirschner, Irina Mader, Jan-Bernd Hövener, Jochen Reiss, José Angel Santamaria-Araujo, Günter Schwarz, Sarah Catharina Grünert. A mild case of molybdenum cofactor deficiency defines an alternative route of MOCS1 protein maturation.
Journal of inherited metabolic disease.
2018 03; 41(2):187-196. doi:
10.1007/s10545-018-0138-7. [PMID: 29368224] - Joanna Jakubiczka-Smorag, Jose Angel Santamaria-Araujo, Imke Metz, Avadh Kumar, Samy Hakroush, Wolfgang Brueck, Guenter Schwarz, Peter Burfeind, Jochen Reiss, Lukasz Smorag. Mouse model for molybdenum cofactor deficiency type B recapitulates the phenotype observed in molybdenum cofactor deficient patients.
Human genetics.
2016 07; 135(7):813-26. doi:
10.1007/s00439-016-1676-4. [PMID: 27138983] - Philippa B Mills, Emma J Footitt, Serkan Ceyhan, Paula J Waters, Cornelis Jakobs, Peter T Clayton, Eduard A Struys. Urinary AASA excretion is elevated in patients with molybdenum cofactor deficiency and isolated sulphite oxidase deficiency.
Journal of inherited metabolic disease.
2012 Nov; 35(6):1031-6. doi:
10.1007/s10545-012-9466-1. [PMID: 22403017] - L H Ngu, B Afroze, B C Chen, O Affandi, M Y Zabedah. Molybdenum cofactor deficiency in a Malaysian child.
Singapore medical journal.
2009 Oct; 50(10):e365-7. doi:
. [PMID: 19907877] - M Arenas, L D Fairbanks, K Vijayakumar, L Carr, E Escuredo, A M Marinaki. An unusual genetic variant in the MOCS1 gene leads to complete missplicing of an alternatively spliced exon in a patient with molybdenum cofactor deficiency.
Journal of inherited metabolic disease.
2009 Aug; 32(4):560-9. doi:
10.1007/s10545-009-1151-7. [PMID: 19544009] - Birthe Smolinsky, Sabrina A Eichler, Sabine Buchmeier, Jochen C Meier, Guenter Schwarz. Splice-specific functions of gephyrin in molybdenum cofactor biosynthesis.
The Journal of biological chemistry.
2008 Jun; 283(25):17370-9. doi:
10.1074/jbc.m800985200. [PMID: 18411266] - Hava Peretz, Meirav Shtauber Naamati, David Levartovsky, Ayala Lagziel, Esther Shani, Ivona Horn, Hanna Shalev, Daniel Landau. Identification and characterization of the first mutation (Arg776Cys) in the C-terminal domain of the Human Molybdenum Cofactor Sulfurase (HMCS) associated with type II classical xanthinuria.
Molecular genetics and metabolism.
2007 May; 91(1):23-9. doi:
10.1016/j.ymgme.2007.02.005. [PMID: 17368066] - K Ichida, H Ibrahim Aydin, M Hosoyamada, H Serap Kalkanoglu, A Dursun, I Ohno, T Coskun, A Tokatli, T Shibasaki, T Hosoya. A Turkish case with molybdenum cofactor deficiency.
Nucleosides, nucleotides & nucleic acids.
2006; 25(9-11):1087-91. doi:
10.1080/15257770600894022. [PMID: 17065069] - A Macaya, L Brunso, N Fernández-Castillo, J A Arranz, H B Ginjaar, E Cuenca-León, R Corominas, M Roig, B Cormand. Molybdenum cofactor deficiency presenting as neonatal hyperekplexia: a clinical, biochemical and genetic study.
Neuropediatrics.
2005 Dec; 36(6):389-94. doi:
10.1055/s-2005-872877. [PMID: 16429380] - M Z Seidahmed, E A Alyamani, M S Rashed, A A Saadallah, O B Abdelbasit, M M Shaheed, A Rasheed, F A Hamid, M A Sabry. Total truncation of the molybdopterin/dimerization domains of SUOX protein in an Arab family with isolated sulfite oxidase deficiency.
American journal of medical genetics. Part A.
2005 Jul; 136(2):205-9. doi:
10.1002/ajmg.a.30796. [PMID: 15952210] - Mohamed S Rashed, Amal A A Saadallah, Zuhair Rahbeeni, Wafaa Eyaid, Mohamed Z Seidahmed, Saad Al-Shahwan, Mustafa A M Salih, Mohammad E Osman, Mohamed Al-Amoudi, Lujane Al-Ahaidib, Minnie Jacob. Determination of urinary S-sulphocysteine, xanthine and hypoxanthine by liquid chromatography-electrospray tandem mass spectrometry.
Biomedical chromatography : BMC.
2005 Apr; 19(3):223-30. doi:
10.1002/bmc.439. [PMID: 15558695] - Munehiro Yoshida. [Molybdenum].
Nihon rinsho. Japanese journal of clinical medicine.
2004 Dec; 62 Suppl 12(?):319-21. doi:
NULL. [PMID: 15658328] - Kimiyoshi Ichida. [Hereditary xanthinuria and molybdenum cofactor deficiency].
Nihon rinsho. Japanese journal of clinical medicine.
2003 Jan; 61 Suppl 1(?):377-82. doi:
NULL. [PMID: 12629751] - J O Sass, M Kishikawa, R Puttinger, J Reiss, W Erwa, A Shimizu, W Sperl. Hypohomocysteinaemia and highly increased proportion of S-sulfonated plasma transthyretin in molybdenum cofactor deficiency.
Journal of inherited metabolic disease.
2003; 26(1):80-2. doi:
10.1023/a:1024091900547. [PMID: 12872846] - Kimiyoshi Ichida. [Primary underproductive hypouricemia].
Nihon rinsho. Japanese journal of clinical medicine.
2003 Jan; 61 Suppl 1(?):364-7. doi:
NULL. [PMID: 12629748] - Heon-Jin Lee, Ibrahim M Adham, Günter Schwarz, Matthias Kneussel, Jörn O Sass, Wolfgang Engel, Jochen Reiss. Molybdenum cofactor-deficient mice resemble the phenotype of human patients.
Human molecular genetics.
2002 Dec; 11(26):3309-17. doi:
10.1093/hmg/11.26.3309. [PMID: 12471057] - Masahiko Kishikawa, Jörn Oliver Sass, Nobuo Sakura, Toyofumi Nakanishi, Akira Shimizu, Masanori Yoshioka. The peak height ratio of S-sulfonated transthyretin and other oxidized isoforms as a marker for molybdenum cofactor deficiency, measured by electrospray ionization mass spectrometry.
Biochimica et biophysica acta.
2002 Nov; 1588(2):135-8. doi:
10.1016/s0925-4439(02)00156-4. [PMID: 12385777] - S Arslanoglu, M Yalaz, D Gökşen, M Coker, S Tütüncüoglu, M Akisu, S Darcan, N Kultursay, M Ciriş, E Demirtaş. Molybdenum cofactor deficiency associated with Dandy-Walker complex.
Brain & development.
2001 Dec; 23(8):815-8. doi:
10.1016/s0387-7604(01)00316-3. [PMID: 11720800] - P B Kang, J V Hunter, E M Kaye. Lactic acid elevation in extramitochondrial childhood neurodegenerative diseases.
Journal of child neurology.
2001 Sep; 16(9):657-60. doi:
10.1177/088307380101600906. [PMID: 11575606] - M Topcu, T Coskun, G Haliloglu, I Saatci. Molybdenum cofactor deficiency: report of three cases presenting as hypoxic-ischemic encephalopathy.
Journal of child neurology.
2001 Apr; 16(4):264-70. doi:
10.1177/088307380101600406. [PMID: 11332461] - S Leimkühler, K V Rajagopalan. In vitro incorporation of nascent molybdenum cofactor into human sulfite oxidase.
The Journal of biological chemistry.
2001 Jan; 276(3):1837-44. doi:
10.1074/jbc.m007304200. [PMID: 11042213] - V V Pollock, M J Barber. Serine 121 is an essential amino acid for biotin sulfoxide reductase functionality.
The Journal of biological chemistry.
2000 Nov; 275(45):35086-90. doi:
10.1074/jbc.m006872200. [PMID: 10948204] - T Watanabe, N Ihara, T Itoh, T Fujita, Y Sugimoto. Deletion mutation in Drosophila ma-l homologous, putative molybdopterin cofactor sulfurase gene is associated with bovine xanthinuria type II.
The Journal of biological chemistry.
2000 Jul; 275(29):21789-92. doi:
10.1074/jbc.c000230200. [PMID: 10801779] - M Kishikawa. [Diagnosis of neurodegenerative disease by mass spectrometry].
Rinsho byori. The Japanese journal of clinical pathology.
2000 May; 48(5):430-6. doi:
NULL. [PMID: 10892291] - M Kishikawa, T Nakanishi, A Shimizu, M Yoshino. Detection by mass spectrometry of highly increased amount of S-sulfonated transthyretin in serum from a patient with molybdenum cofactor deficiency.
Pediatric research.
2000 Apr; 47(4 Pt 1):492-4. doi:
10.1203/00006450-200004000-00013. [PMID: 10759156] - F M Carragher, J M Kirk, C Steer, J Allen, C Dorche. False negative thiosulphate screening test in a case of molybdenum cofactor deficiency.
Journal of inherited metabolic disease.
1999 Oct; 22(7):842-3. doi:
10.1023/a:1005518426781. [PMID: 10518287] - R U Meckenstock, R Krieger, S Ensign, P M Kroneck, B Schink. Acetylene hydratase of Pelobacter acetylenicus. Molecular and spectroscopic properties of the tungsten iron-sulfur enzyme.
European journal of biochemistry.
1999 Aug; 264(1):176-82. doi:
10.1046/j.1432-1327.1999.00600.x. [PMID: 10447686] - W S Waring, S Maxwell. Diagnosis of molybdenum cofactor deficiency.
Lancet (London, England).
1999 Feb; 353(9153):675-6. doi:
10.1016/s0140-6736(05)75474-x. [PMID: 10030364] - H A Simmonds, G F Hoffmann, J L Pérignon, V Micheli, A H van Gennip. Diagnosis of molybdenum cofactor deficiency.
Lancet (London, England).
1999 Feb; 353(9153):675. doi:
10.1016/s0140-6736(05)75473-8. [PMID: 10030363] - H Koch. Dipsticks and convulsions.
Lancet (London, England).
1998 Dec; 352(9143):1824. doi:
10.1016/s0140-6736(98)09360-x. [PMID: 9851384] - J Vion-Dury, A M Salvan, S Confort-Gouny, P J Cozzone. Atlas of brain proton magnetic resonance spectra. Part II: Inherited metabolic encephalopathies.
Journal of neuroradiology = Journal de neuroradiologie.
1998 Dec; 25(4):281-9. doi:
NULL. [PMID: 10048272] - W D Graf, O E Oleinik, R M Jack, A H Weiss, J L Johnson. Ahomocysteinemia in molybdenum cofactor deficiency.
Neurology.
1998 Sep; 51(3):860-2. doi:
10.1212/wnl.51.3.860. [PMID: 9748040] - F Blasco, J P Dos Santos, A Magalon, C Frixon, B Guigliarelli, C L Santini, G Giordano. NarJ is a specific chaperone required for molybdenum cofactor assembly in nitrate reductase A of Escherichia coli.
Molecular microbiology.
1998 May; 28(3):435-47. doi:
10.1046/j.1365-2958.1998.00795.x. [PMID: 9632249] - K Ichida, T Hosoya. [Classical xanthinuria (type I and II)].
Ryoikibetsu shokogun shirizu.
1998; ?(18 Pt 1):470-3. doi:
NULL. [PMID: 9590104] - T Matsuishi, S Ishibashi, M Nakashima, M Satoi. [Molybdenum cofactor deficiency].
Ryoikibetsu shokogun shirizu.
1998; ?(19 Pt 2):185-8. doi:
NULL. [PMID: 9645039] - K Ichida, Y Amaya, N Kamatani, T Nishino, T Hosoya, O Sakai. Identification of two mutations in human xanthine dehydrogenase gene responsible for classical type I xanthinuria.
The Journal of clinical investigation.
1997 May; 99(10):2391-7. doi:
10.1172/jci119421. [PMID: 9153281] - S Sumi, Y Wada. [Xanthine oxidase deficiency (hereditary xanthinuria), molybdenum cofactor deficiency].
Nihon rinsho. Japanese journal of clinical medicine.
1996 Dec; 54(12):3333-6. doi:
. [PMID: 8976115] - N Blau, J B de Klerk, B Thöny, C W Heizmann, L Kierat, J A Smeitink, M Duran. Tetrahydrobiopterin loading test in xanthine dehydrogenase and molybdenum cofactor deficiencies.
Biochemical and molecular medicine.
1996 Aug; 58(2):199-203. doi:
10.1006/bmme.1996.0049. [PMID: 8812740] - E Bonioli, A DiStefano, A Palmieri, A Bertola, C Bellini, U Caruso, A R Fantasia, G Minniti, C Dorche. Combined deficiency of xanthine oxidase and sulphite oxidase due to a deficiency of molybdenum cofactor.
Journal of inherited metabolic disease.
1996; 19(5):700-1. doi:
10.1007/bf01799850. [PMID: 8892030] - Z M Habbal, E H Touma. Positive interference from homocystinuria urine in a spot test for molybdenum cofactor deficiency.
Clinical chemistry.
1995 Jul; 41(7):1056-7. doi:
10.1093/clinchem/41.7.1056. [PMID: 7600694] - C Mize, J L Johnson, K V Rajagopalan. Defective molybdopterin biosynthesis: clinical heterogeneity associated with molybdenum cofactor deficiency.
Journal of inherited metabolic disease.
1995; 18(3):283-90. doi:
10.1007/bf00710416. [PMID: 7474893] - G Pintos-Morell, M A Naranjo, M Artigas, M Roge, M Rodes, M J Coll, J L Johnson, K V Rajagopalan. Molybdenum cofactor deficiency associated with Dandy-Walker malformation.
Journal of inherited metabolic disease.
1995; 18(1):86-7. doi:
10.1007/bf00711384. [PMID: 7623453] - J L Johnson, K V Rajagopalan. An HPLC assay for detection of elevated urinary S-sulphocysteine, a metabolic marker of sulphite oxidase deficiency.
Journal of inherited metabolic disease.
1995; 18(1):40-7. doi:
10.1007/bf00711371. [PMID: 7623441] - M Volk, O Meyer, K Frunzke. Metabolic relationship between the CO dehydrogenase molybdenum cofactor and the excretion of urothione by Hydrogenophaga pseudoflava.
European journal of biochemistry.
1994 Nov; 225(3):1063-71. doi:
10.1111/j.1432-1033.1994.1063b.x. [PMID: 7957196] - A H van Gennip, N G Abeling, A E Stroomer, H Overmars, H D Bakker. The detection of molybdenum cofactor deficiency: clinical symptomatology and urinary metabolite profile.
Journal of inherited metabolic disease.
1994; 17(1):142-5. doi:
10.1007/bf00735420. [PMID: 8051926] - A H van Gennip, H Mandel, L E Stroomer, A G van Cruchten. Effect of allopurinol on the xanthinuria in a patient with molybdenum cofactor deficiency.
Advances in experimental medicine and biology.
1994; 370(?):375-8. doi:
10.1007/978-1-4615-2584-4_81. [PMID: 7660932] - G Mann, J M Kirk. Antibiotic interference in urinary thiosulphate measurements.
Journal of inherited metabolic disease.
1994; 17(1):120-1. doi:
10.1007/bf00735412. [PMID: 8051920] - R G Boles, L R Ment, M S Meyn, A L Horwich, L E Kratz, P Rinaldo. Short-term response to dietary therapy in molybdenum cofactor deficiency.
Annals of neurology.
1993 Nov; 34(5):742-4. doi:
10.1002/ana.410340520. [PMID: 7694543] - G L Arnold, C L Greene, J P Stout, S I Goodman. Molybdenum cofactor deficiency.
The Journal of pediatrics.
1993 Oct; 123(4):595-8. doi:
10.1016/s0022-3476(05)80961-8. [PMID: 8410516] - L K Hansen, K Wulff, C Dorche, E Christensen. Molybdenum cofactor deficiency in two siblings: diagnostic difficulties.
European journal of pediatrics.
1993 Aug; 152(8):662-4. doi:
10.1007/bf01955243. [PMID: 8404970] - H M Slot, W C Overweg-Plandsoen, H D Bakker, N G Abeling, P Tamminga, P G Barth, A H Van Gennip. Molybdenum-cofactor deficiency: an easily missed cause of neonatal convulsions.
Neuropediatrics.
1993 Jun; 24(3):139-42. doi:
10.1055/s-2008-1071531. [PMID: 8355818] - J L Johnson, K V Rajagopalan, S K Wadman. Human molybdenum cofactor deficiency.
Advances in experimental medicine and biology.
1993; 338(?):373-8. doi:
10.1007/978-1-4615-2960-6_77. [PMID: 8304141] - A H van Gennip, A Stroomer, W G Plandsoen, N G Abeling. The effect of molybdenum cofactor deficiency on the purine pattern of cerebrospinal fluid.
Journal of inherited metabolic disease.
1991; 14(3):364-6. doi:
10.1007/bf01811704. [PMID: 1770793] - A P Reynolds, R A Harkness. Urinary thiosulphate/creatinine concentration ratio in hospitalized children.
Journal of inherited metabolic disease.
1991; 14(6):938-9. doi:
10.1007/bf01800478. [PMID: 1779654] - F J Bamforth, J L Johnson, A G Davidson, L T Wong, G Lockitch, D A Applegarth. Biochemical investigation of a child with molybdenum cofactor deficiency.
Clinical biochemistry.
1990 Dec; 23(6):537-42. doi:
10.1016/0009-9120(90)80046-l. [PMID: 2289312] - C Iobbi-Nivol, C L Santini, F Blasco, G Giordano. Purification and further characterization of the second nitrate reductase of Escherichia coli K12.
European journal of biochemistry.
1990 Mar; 188(3):679-87. doi:
10.1111/j.1432-1033.1990.tb15450.x. [PMID: 2139607] - J L Johnson, M M Wuebbens, R Mandell, V E Shih. Molybdenum cofactor biosynthesis in humans. Identification of two complementation groups of cofactor-deficient patients and preliminary characterization of a diffusible molybdopterin precursor.
The Journal of clinical investigation.
1989 Mar; 83(3):897-903. doi:
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European journal of pediatrics.
1988 Dec; 148(3):246-9. doi:
10.1007/bf00441412. [PMID: 3215199] - A Aukett, M J Bennett, G P Hosking. Molybdenum co-factor deficiency: an easily missed inborn error of metabolism.
Developmental medicine and child neurology.
1988 Aug; 30(4):531-5. doi:
10.1111/j.1469-8749.1988.tb04781.x. [PMID: 3169394] - J L Johnson, M M Wuebbens, R Mandell, V E Shih. Molybdenum cofactor deficiency in a patient previously characterized as deficient in sulfite oxidase.
Biochemical medicine and metabolic biology.
1988 Aug; 40(1):86-93. doi:
10.1016/0885-4505(88)90108-9. [PMID: 3219233] - J L Johnson. Trimethylamine oxidation in liver tissue is not catalyzed by a molybdenum cofactor-dependent enzyme.
BioFactors (Oxford, England).
1988 Jul; 1(2):153-5. doi:
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Biochimica et biophysica acta.
1988 Apr; 954(1):1-13. doi:
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Annales de pediatrie.
1986 Nov; 33(9):825-8. doi:
. [PMID: 3800248] - F Hervé, J P Berger, J Soulier. [Sulfite and xanthine oxidase deficiency: a diagnosis based on 2 simple tests].
Annales de pediatrie.
1986 Nov; 33(9):857. doi:
NULL. [PMID: 3800254] - R A Roesel, F Bowyer, P R Blankenship, F A Hommes. Combined xanthine and sulphite oxidase defect due to a deficiency of molybdenum cofactor.
Journal of inherited metabolic disease.
1986; 9(4):343-7. doi:
10.1007/bf01800483. [PMID: 3104671] - A Roth, C Nogues, J P Monnet, H Ogier, J M Saudubray. Anatomo-pathological findings in a case of combined deficiency of sulphite oxidase and xanthine oxidase with a defect of molybdenum cofactor.
Virchows Archiv. A, Pathological anatomy and histopathology.
1985; 405(3):379-86. doi:
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Journal of inherited metabolic disease.
1983; 6 Suppl 1(?):78-83. doi:
10.1007/bf01811328. [PMID: 6413778] - J L Johnson, K V Rajagopalan. Structural and metabolic relationship between the molybdenum cofactor and urothione.
Proceedings of the National Academy of Sciences of the United States of America.
1982 Nov; 79(22):6856-60. doi:
10.1073/pnas.79.22.6856. [PMID: 6960353] - J L Johnson, W R Waud, K V Rajagopalan, M Duran, F A Beemer, S K Wadman. Inborn errors of molybdenum metabolism: combined deficiencies of sulfite oxidase and xanthine dehydrogenase in a patient lacking the molybdenum cofactor.
Proceedings of the National Academy of Sciences of the United States of America.
1980 Jun; 77(6):3715-9. doi:
10.1073/pnas.77.6.3715. [PMID: 6997882]